Cone Beam-CT-Based Bone Volume Assessments of Alveolar Synthetic Bone Graft GlassBONE™ in Cleft Lip and Palate Patients: A Retrospective Study.

Background: Clefts of the lip and palate (CLP) are facial deformities that require multiple surgical procedures during childhood. One of these steps consists of filling the alveolar space with bone graft, traditionally removed from the iliac crest. However, this procedure could be invasive in children. Aim: Here, we aimed to evaluate the outcomes of GlassBONE™ graft, a bioactive glass used as a bone substitute, as an alternative to the deleterious autologous bone graft in children. Materials & methods: Retrospective monocentric study with 17 children aged 7.5 ± 2.2 yo [3.8-13.3 yo] carrying CLP. This technique has been established at La Timone Children hospital (Assistance Publique - Hôpitaux de Marseille) since 2011. Clinical (scar, graft rejection and periodontal status) and radiological (both panoramic radiographs and cone beam-CT) follow-up was conducted one year after the graft. The primary outcome was the reduction of the cleft volume, and secondary was the eruption of the adjacent tooth through the graft. Results: GlassBONE™ permitted a significant reduction in the cleft volume by 42.4 ± 27.7% [0.6-81.1%] (p < 0.0001), corresponding to a filling of 57.6 ± 27.7% of the alveolar cleft. GlassBONE™ is well tolerated, ensuring satifactory clinical results (improvement in both scar and periodontal coverage), as well as the physiological evolution of the germs through the biomaterial. GlassBONE™ appears particularly suitable for small volumes, and we were able to determine a minimum volume of approximtely 0.259 + / - 0.155 cc required for a successful bone fusion. Conclusion: The bioactive glass GlassBONE™ could be safely used in children with small CLP cases, providing satisfactory clinical and radiological results.

A Comparative In Vitro and In Vivo Study of Osteogenicity by Using Two Biomaterials and Two Human Mesenchymal Stem Cell Subtypes.

Background: Bone repair induced by stem cells and biomaterials may represent an alternative to autologous bone grafting. Mesenchymal stromal/stem cells (MSCs), easily accessible in every human, are prototypical cells that can be tested, alone or with a biomaterial, for creating new osteoblasts. The aim of this study was to compare the efficiency of two biomaterials-biphasic calcium phosphate (BCP) and bioactive glass (BG)-when loaded with either adult bone marrow mesenchymal stem cells (BMMSCs) or newborn nasal ecto-mesenchymal stem cells (NE-MSCs), the latter being collected for further repair of lip cleft-associated bone loss. Materials and Methods: BMMSCs were collected from two adults and NE-MSCs from two newborn infants. An in vitro study was performed in order to determine the best experimental conditions for adhesion, viability, proliferation and osteoblastic differentiation on BCP or BG granules. Bone-associated morphological changes and gene expression modifications were quantified using histological and molecular techniques. The in vivo study was based on the subcutaneous implantation in nude mice of the biomaterials, loaded or not with one of the two cell types. Eight weeks after, bone formation was assessed using histological and electron microscopy techniques. Results: Both cell types-BMMSC and NE-MSC-display the typical stem cell surface markers-CD73+, CD90+, CD105+, nestin - and exhibit the MSC-associated osteogenic, chondrogenic and adipogenic multipotency. NE-MSCs produce less collagen and alkaline phosphatase than BMMSCs. At the transcript level, NE-MSCs express more abundantly three genes coding for bone sialoprotein, osteocalcin and osteopontin while BMMSCs produce extra copies of RunX2. BMMSCs and NE-MSCs adhere and survive on BCP and BG. In vivo experiments reveal that bone formation is only observed with BMMSCs transplanted on BCP biomaterial. Conclusion: Although belonging to the same superfamily of mesenchymal stem cells, BMMSCs and NE-MSCs exhibit striking differences, in vitro and in vivo. For future clinical applications, the association of BMMSCs with BCP biomaterial seems to be the most promising.

[The role of prophylactic cranial irradiation in small cell lung cancer]. / Quel rôle pour l'irradiation cérébrale prophylactique dans le cancer à petites cellules ? Une étude rétrospective unicentrique.

INTRODUCTION: Prophylactic cranial irradiation (PCI) is considered standard therapeutic management in small cell lung cancer (SCLC). This is based on old randomised trials with methodological limitations, namely the absence of magnetic resonance imaging (MRI) of the brain. The aim of this study is to assess the risk not administering PCI when systematic brain imaging is applied. METHODS: Retrospective study including untreated SCLC, without PCI and receiving brain imaging at the time of diagnosis. Kaplan-Meier and log-rank statistics were used for survival analyses. RESULTS: Among 150 patients, 75 were possibly eligible for PCI. Thirteen patients presented with an isolated brain recurrence as the first site of progression with no other metastatic sites apparent, and in 6 patients, the brain was the only recurrent site during the whole follow-up. In the group of patients eligible for PCI, there was no statistically significant survival difference according to the brain progression status (P=0.11). CONCLUSIONS: The expected impact of PCI seems limited in terms of overall survival and prevention of isolated brain metastases in patients having systematic brain imaging during SCLC work-up.

C1-C2 type Harms internal fixation for unstable C2 fracture in a 6-year-old boy: Case report.

BACKGROUND: Decision-making is often difficult in odontoid fracture in children. CASE REPORT: We present the case of a 6-year-old boy who sustained cervical trauma on falling out of a tree. Initial cervical X-ray and CT-scan did not find any traumatic lesion. Three-week check-up revealed an unstable C2 fracture in the synchondrosis at the base of the odontoid bone, with anterior displacement (type IC on the classification of Hosalkar et al.), without neurological symptoms except for cervical pain and limitation of head rotation. MRI confirmed the absence of medullary lesion. The Harms technique was used to fix C1 and C2, using adult instrumentation without bone graft. Bone fusion was obtained at 8 months. Hardware was removed at 10 months. No complications were reported. CONCLUSIONS: Posterior internal fixation for unstable C2 fractures in children can be effective and relatively safe.

Multiple neurosurgical treatments for different members of the same family with Currarino syndrome.

INTRODUCTION: Currarino's syndrome (CS) is an autosomal dominant disorder of embryonic development causing a rare malformating syndrome characterized by a triad of an anorectal malformations, presacral mass (most commonly an anterior sacral meningocele) and sacral bony defects. Mutations of the HLXB9 gene have been identified in most CS cases, but a precise genotype-phenotype correlation has not been described so far. Family screening is obligatory. The diagnosis is usually made during childhood and rarely in adulthood. In this context, imaging, and especially MRI plays a major role in the diagnosis of this syndrome. Surgical management is provided by pediatric surgeons or neurosurgeons. FAMILIAL CASE REPORT: Here, we present a family case report with CS requiring different neurosurgical management. The son, a 3-year-old boy, developed a tethered spinal cord syndrome associated to a lipoma of the filum terminale, a sacro-coccygeal teratoma and an anal adhesion. A combined surgical approach permitted a good evolution on the urinary and digestive functions despite a persistent fecal incontinence. The 2-year-old daughter presented with a cyst of the thyreoglossal tract infected and fistulized to the skin. She was also followed for a very small lipoma of the filum terminale that required a neurosurgical approach. The father, 44-year-old, manifested functional digestive and urinary disorders caused by a giant anterior sacral meningocele. The ligation of the neck of the cyst and aspiration of the liquid inside in full through a posterior partial approach permit a complete collapse of the cyst with an instantly satisfactory clinical outcome. CONCLUSION: In these cases, cooperation between pediatric surgeons and neurosurgeons was crucial. The follow-up of these patients should be done in a spina bifida clinic. A geneticist evaluation must be offered to the patient in the case of a CS as well as a clinical evaluation of the relatives (parents, siblings).

[Gluten: Is the information available on the Internet valid?] / Gluten : l'information disponible sur Internet est-elle en accord avec les recommandations des sociétés savantes ?

BACKGROUND: Internet provides easy access to health information, but the quality and validity of this information vary. OBJECTIVES: Evaluate the quality of website structures and the information provided on celiac disease (CD), gluten sensitivity (GS), and wheat allergy (WA). MATERIALS AND METHODS: The websites addressing CD, GS, and WA appearing on the first two pages of Google, Yahoo, and Bing from seven selected queries were investigated. We initially assessed the website structures with one instrument (Netscoring) and the presence of certification (quality label Health On the Net (HON code)). Then we evaluated the content of each website concerning the information about CD, GS, and WA. Our repository was based on the most recent guidelines of the European Society of Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) and the World Gastroenterology Organization (WGO) published in 2012. The websites were classified into eight categories. RESULTS: One hundred and five websites were included. Twenty-one websites obtained a sufficient score with the Netscoring instrument (average 113.6/312). There was a significant correlation between the referenced websites analyzed and the grades obtained with the Netscoring instrument (Pearson=0.39, P=0.2×10-5): websites of scientific societies (11.8/18), community websites (9.44/18), and website associations (9.4/18). There was a significant correlation between the results obtained for the websites on CD, GS, and WA and the results obtained for the websites with the Netscoring instruments (Pearson=0.41, P=2.6×10-6). Only three websites were consistent with the guidelines on CD, GS, and WA. CONCLUSION: The websites were partially in agreement with the guidelines. To date, the pediatrician remains the main actor in parental guidance concerning gluten information.

[Management of pediatric liver abscess]. / Abcès hépatique chez l'enfant : à propos d'un cas.

Liver abscess in the pediatric population remains uncommon in developed countries, except in cases of septicemia or in children with major debilitating diseases, granulocyte dysfunction, or immunosuppression. Although much is known about the etiopathogenesis of liver abscess, the gold standard of investigations and treatment is still debatable in developing countries. We report the case of a 6-year-old child living in Reunion Island, with no medical history, presenting with right and pyretic abdominal pain in the right upper quadrant. Ultrasound and CT scan showed a large hypodense nonenhanced area in segment IV. Final diagnosis was, by exclusion, pyogenic liver abscess based on negative serology, recent liver lesion, and normal tumor test results, even if blood culture remained negative. No percutaneous puncture was done because of positive outcome after 4 days of antibiotics. Treatment consisted in three intravenous antibiotics (ceftriaxone, aminoxide, and metronidazole) until complete biological normalization. Ultrasound remained normal 3 months later. Even if liver abscess is uncommon in developing countries, the diagnosis must be raised in cases of isolated liver tumor with fever. Management in the nonimmunosuppressed child must be discussed associating parenteral antibiotic therapy, percutaneous drainage, or surgery in very uncommon cases, according to the liver location and first day's progression. Etiological investigation such as colonoscopy in adults must be adapted to pediatric data.

Penile length is comparable in boys with and without hypospadias.

INTRODUCTION: It has been suggested that penile length is negatively correlated to the degree of hypospadias; however, there are no studies in the literature actually comparing penile length in normal patients and patients with hypospadias. MATERIAL AND METHOD: Between January and May 2011, we measured penile length in two groups of children aged up to 5 years. The first group comprised all boys admitted for hypospadias (40 patients; 25 distal and 15 proximal hypospadias). The control group comprised 100 boys seen for other surgical procedures excluding those with endocrine disorders. We measured the length of the dorsal aspect of the penis in the flaccid state, without stretching. We then established, using our control group, a range of 'normal' values for penile length in our population and determined whether children with hypospadias were within this 'normal' range. RESULTS: Mean penile length in the control group was 4.07 ± 0.92 cm, mean penile length in the hypospadias group was 4.36 ± 0.9 cm (4.48 ± 0.89 cm for distal hypospadias and 4.21 ± 0.79 cm for proximal hypospadias). There was no difference in penile length between the hypospadias group and the control group regardless of degree of hypospadias. CONCLUSION: This study indicates that penile length in children with hypospadias whether proximal or distal is within the normal range.

[Pelvic kystic schwannoma evoking a tumor of the right seminal vesicle]. / Schwannome kystique pelvien évoquant une tumeur de la vésicule séminale droite.

We present the case of a pelvic schwannoma in a 36 year old man. It was discovered by chance during the medical assessment of a prostatitis. A computed tomography scan and magnetic resonance imaging revealed a 8 centimeters cystic pelvic tumor, closed to the right seminal vesicle. The patient underwent a transrectal ultrasound-guided biopsy, which showed a proliferation of neural peripheric cells with nuclear abnormalities. A tumorectomy was performed by laparotomy. The histological study diagnosed a benign cystic schwannoma. The patient was free of disease 12 months postoperatively.